We report a rare case of a 21-year-old male who had myxoma originating from septal band of the right ventricle. He was otherwise healthy young man without any associated symptom. His cardiac murmur was found incidentally upon routine health control mass examination. Subsequently, echocardiography and angiocardiography demonstrated a tumor occupying the right ventricular outflow tract and protruding into the main pulmonary artery in systolic phase. He was operated upon using extracorporeal circulation. By right ventriculotomy the tumor was resected together with septal tissue 5 mm around the stalk, which branched into the myocardium. Resected mass was 4 x 3.7 cm in size and 5 grams in weight. Because of the frozen section of the surgical stump was positive for tumor cells, additional resection of septal myocardium was carried out. The defect was closed using mattress sutures with dacron felt strips. His postoperative course was uneventful. Thirty-two months after the surgery, patient has no sign of recurrence. Right ventricular myxoma is uncommon with only 27 cases appearing in Japanese literature. In review of literature, we discussed the characteristics and management of the right ventricular myxoma.